Is your blood boiling? Let's learn about Polycythemia Vera (PV) in simple terms

Do you often feel dizzy for no reason? Do you feel very tired? Do you itch unbearably, especially after a hot bath? Do you hear a ringing in your ears? We often think that these things are normal and ignore them. But sometimes, behind these minor symptoms, there may be a condition that we have not heard much about and that we should be concerned about. Today we are going to talk about one such disease. That is Polycythemia Vera , or PV for short.

Simply put, what is polycythemia vera (PV)?

Simply put, polycythemia vera (PV) is a blood disorder in our bodies. It causes our bodies to produce too many red blood cells . Now you might be thinking, "Red blood cells are good for the body, so what happens when we produce too many?" That's the problem.

Imagine that there is a road that can carry a certain number of vehicles. What would happen if you put a thousand times more vehicles on that road than it can carry? The entire road would be blocked, and the vehicles would not be able to move, right? That's what happens inside our blood vessels. When there are too many red blood cells, our blood thickens . Just like something that should be water thickens like honey.

This thickening of the blood causes the blood to flow very slowly. This increases the risk of blood clots . If this blood clot travels to the heart, it can cause a heart attack, and if it travels to the brain, it can cause a stroke. Not only that, but it can also cause annoying symptoms such as constant itching, ringing in the ears, stomach pain, nosebleeds, and blurred vision.

Is this really cancer? Should I be worried?

This question is something that many people have. Yes, polycythemia vera is a type of blood cancer. Medically, we call it Myeloproliferative Neoplasm (MPN) .

Now, when we hear the word "cancer," we get very scared. That's normal. But we need to understand that not all cancers are the same. Some are very fast-growing and aggressive. But PV is a very slow-growing type of cancer. That means it doesn't get aggressive all at once. Most of the time, this disease is diagnosed after the age of 60.

Rather than dying from this disease itself, the risk is greater from complications such as heart attacks and strokes caused by blood clots, which we discussed earlier. Also, very rarely, this condition can turn into another serious type of blood cancer. But that happens to a very small percentage. So, even though it's called "cancer," this is a condition that can be managed well with proper treatment.

How does this disease affect our bodies and what are the risks?

Let's now see how this increase in red blood cells affects different parts of our body.

• Blood clotting: This is the main problem. When blood clots, the heart has to work harder to pump blood. Also, blood flow through the body's delicate vessels decreases. This means that our organs and tissues receive less oxygen .
• Spleen Overwork: One of the functions of our body's organ called the spleen is to filter out old, damaged blood cells. Now, when so many red blood cells are produced, the spleen becomes overloaded. It's like trying to filter a lot of dirt through a single filter and it gets clogged. This can cause the spleen to swell. We call it splenomegaly . Then you may feel a heaviness and pain in the left side of your abdomen.

Let's look at the table below to see what the main risks and complications of this PV condition may be.

What are the cases of this disease?

PV usually develops slowly over several stages.

1. Early PV: At this stage, most people have no symptoms at all . Or they only have very mild symptoms, such as dizziness and fatigue. It is sometimes discovered incidentally when a blood test is done for another condition.

2. Advancing PV: Symptoms gradually increase over time. Symptoms such as itching, weight loss, and abdominal discomfort due to an enlarged spleen may occur during this stage.

3. The final stage ("Spent Phase"): This is a bit more complicated. After many years, the abnormal cells that caused the disease spread throughout our bone marrow, damaging the bone marrow. The bone marrow is like the factory where our blood cells are made. When this factory is damaged, scar tissue forms in it. Then, healthy blood cells cannot be made. Surprisingly, the disease that started with the production of too many red blood cells eventually leads to a condition where there are not enough red blood cells, which is anemia .

What are the symptoms that can be identified?

The symptoms of PV often develop slowly over time. Some people can go for years without any symptoms. Let's take a look at what these symptoms are.

Why is this happening? What is the reason?

The main reason for this is a gene mutation in a stem cell in our bone marrow. It's like one line of code in a computer program goes wrong. Then the entire program goes haywire.

There are genes that tell our blood cells to grow. More than 90% of PV patients have a mutation in a gene called JAK2 . Because of this faulty instruction, the bone marrow gets the command, "Keep making red blood cells, don't stop." So red blood cells are produced out of control.

How does a doctor accurately diagnose this disease?

If you have these symptoms, your doctor will first ask you about your symptoms. Then they will do some tests to confirm the diagnosis. According to the World Health Organization (WHO), three criteria must be met to diagnose PV.

1. Blood Tests: This is the first and most important test. It checks to see if your red blood cell count is too high.

• High hemoglobin levels.
• Increased hematocrit (the percentage of red cells in the blood).

2. Bone Marrow Biopsy: A very small sample of bone marrow is taken from the hip bone and examined under a microscope to see if there are any abnormal cells in the bone marrow.

3. Genetic testing or hormone levels:

• A genetic test is performed to check for the JAK2 gene mutation .
• Or, they look at the level of a hormone called erythropoietin (EPO), which is produced by the kidneys. This hormone signals the bone marrow to make red blood cells. In PV, the body stops producing this hormone because red blood cells are being produced on their own. As a result, EPO levels are very low.

What are the treatments for this?

There are two main goals of treating PV. One is to reduce the risk of blood clots, and the other is to control bothersome symptoms.

• Phlebotomy - Blood removal: This is the most commonly used and simplest treatment. Similar to donating blood, a small amount of blood (usually about a pint) is removed from your body at regular intervals . This can reduce the density of your blood and the number of red blood cells.
• Low-dose aspirin: Your doctor may recommend taking a low-dose aspirin pill daily to prevent blood clots. However, this may not be appropriate if you have problems such as stomach ulcers.
• Red blood cell-reducing drugs: If the disease is severe or if blood transfusions alone are not enough, drugs that reduce red blood cell production are given. Examples include hydroxyurea and interferon alfa .
• Treatment for itching: Antihistamines or other specific medications may be prescribed to control severe itching.

How can I live well with this disease?

Once you know you have this disease, there are many things you can do to live with it.

• See your doctor regularly: Go to clinics on time, get blood tests done. It is very important for your doctor to monitor your condition.
• Exercise: Moderate exercise can help maintain good blood circulation.
• Avoid smoking: Smoking thins blood vessels, which increases the risk of blood clots.
• Avoid environments with low oxygen levels: High places like mountain peaks have low oxygen levels. Avoid going to such places.
• Control blood pressure: Maintaining a healthy body weight can help control blood pressure.
• Drink plenty of water: Dehydration can increase blood clotting. Therefore, drink plenty of water throughout the day.

Although PV is a cancer, many people live well for decades after being diagnosed with the disease. The biggest threat is complications from blood clots, rather than the cancer itself. Therefore, maintaining a regular relationship with your doctor and following a healthy lifestyle are as important as medical treatment for this disease.

Polycythemia vera, PV disease, blood cancer, red blood cell proliferation, blood clotting, JAK2 gene, Sri Lanka Health